By Amy Vanessa Frederick
Globally the numbers of confirmed cases and resulting deaths due to the novel coronavirus, SARS-CoV-2 and the disease it causes, COVID-19 are increasing daily.
In an article in the British Journal of Hematology (BJH), Hussain, F, et al. (BJH, 16734, April 2020) stated that “Severe acute respiratory syndrome coronavirus 2, also known as COVID-19, has spread to 184 countries with almost 1.5 million cases as of mid-April 2020 since first reported”. Currently there are 3,593,627 cases and 249,112 deaths.
They further stated that “the clinical features of this disease are not completely understood. However, severe illness is thought to predominantly occur in adults with advanced age and those with underlying comorbidities.
The Center for Disease Control (CDC) recommends that individuals of any age with blood disorders such as sickle cell disease (SCD) may be at an increased risk of developing serious COVID-19 symptoms. These individuals should follow the guidelines to stay healthy and keep their immune systems strong. The immune systems of individuals with SCD are said to be weakened, so maximizing the prevention to all types of infections is important.
According to the Sickle Cell Disease Foundation (SCDF),“SCD is the most common genetic disease in the world”. They state that currently 250 million people worldwide carry the gene responsible for SCD. The SCDF further states that – “it is commonly found in people whose families come from Africa, South or Central America, Caribbean Islands, Mediterranean countries (such as Turkey, Greece and Italy, India and Saudi Arabia)”. SCD, an immuno-compromised condition, puts patients at higher risk for respiratory infections and subsequent pulmonary complications such as acute chest syndrome (ACS)”.
Red blood cells (RBC) with healthy hemoglobin have a disc-shaped structure. This makes them flexible, allowing them to carry oxygen throughout the bloodstream to organs and tissues. SCD affects the hemoglobin in the RBC; causing normally oval-shaped RBC to assume a sickle shape. Hemoglobin is the protein that carries oxygen throughout the body. The hemoglobin in SCD is known as hemoglobin S, or sickle hemoglobin.
RBC with sickle hemoglobin become rigid, making it difficult for them to get through smaller blood vessels. This prevents or slows blood flow. Poor oxygen delivery can damage major organs, including the brain, eyes, lungs, liver, heart, spleen, and kidneys. SCD also causes inflammation and episodes of sudden and severe pain, known as pain crisis or vaso-occlusive crisis (VOC).
Sickle cells usually have a shorter lifespan than healthyRBC. Their typical lifespan is 10 to 20 days, while the lifespan is 90 to 120 days for normal cells. Although the body is always working to replace red blood cells that die, with sickle cell, it can’t replace them fast enough. This leads to a lower than normal level of the red blood cells, resulting in anemia or tiredness and weakness.
As stated in Hopkinsmedicine.org, people with sickle cell anemia can also have painfully swollen hands and feet, and vision problems. In addition, their growth can be delayed, and they can be more prone to infections. Pain usually disappears between the crises in children with sickle cell anemia, but the period of pain can persist in adolescents and adults.
SCD is a disease with two complex and contributing factors that are connected. Sickle cell impairs the transfer of oxygen through the body by hemoglobin in the red blood cells; which can lead to anemia and vaso-occlusion. SCD can affect many systems such as the cardiopulmonary, musculoskeletal, central nervous system (CNS), gastrointestinal (GI) and the reticuloendothelial (Immune system).
Conversely COVID-19, a respiratory disease, also impairs the transfer of oxygen throughout the body. COVID-19 pathology is associated with reduced cellular oxygenation, pneumonia, and acute respiratory distress syndrome(ARDS) in severe cases, which is a major cause of morbidity and mortality in patients with SCD. This pathological process is closely linked to an increased risk of vaso-occlusive complications in sickle cell disease, including acute chest syndrome.
Hussain et al. stated “we tested 14 SCD patients for COVID-19, four patients, tested positive, all have history of respiratory complications, such as ACS, asthma, orpulmonary embolism (PE), which may be potential risk factors for progressive COVID-19 pulmonary disease in patients with SCD.
All four patients initially presented to the emergency department for typical VOC, and the clinical course of their COVID-19 infection was rather mild considering that these patients were immunocompromised, except for one patient where the ACS episode may or may not have been related to the COVID-19 infection.
Additionally, they stated: “we wonder if the background chronic inflammatory, hemolytic and anemic state in SCD might have a favorable influence in protecting this patient population from fatal COVID-19 infection. Severe respiratory symptoms due to COVID-19 would qualify for the definition of ACS in SCD patients”. They believe that further studies with more patients will be needed for a better understanding of COVID-19 in this (SCD) patient population.
Of interest, although COVID -19 has not infected many children during the period of the pandemic, a report fromReuters on April 30 stated that “some children in the United Kingdom with no underlying health conditions have died from a rare inflammatory syndrome, which researchers believe to be linked to COVID-19”.
Italian and British medical experts are said to be investigating a possible link between the coronavirus pandemic and clusters of severe inflammatory disease among infants who are arriving in hospital with high fevers and swollen arteries.
Doctors in northern Italy, one of the world’s hardest-hit areas during the pandemic, have reported extraordinarily large numbers of children under age 9 with severe cases of what appears to be Kawasaki disease, which is more common in parts of Asia.
“There are some children who have died who didn’t have underlying health conditions,” they reported. “It’s a new disease that we think may be caused by the COVID-19 virus, we’re not 100% sure because some of the people who got it hadn’t tested positive, so we’re doing a lot of research now but it is something that we’re worried about.”
In the United States, the New York Times May 5, 2020 edition stated that 15 children were hospitalized with a “multisystem inflammatory syndrome potentially associated with COVID-19. Many of these children were previously infected with coronavirus in the past.
“In areas with large numbers of individuals with SCD and COVID-19 outbreak, diagnostic and treatment resources should be scaled up along with investment in public health announcements targeted to the at-risk sickle cell disease population. When an effective and affordable COVID-19 treatment becomes available, people with sickle cell disease should be prioritized because of their increased risk of mortality” Dexter, D et.al.; Lancet May 23, 2020.
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